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Juvenile Myasthenia Gravis: An Indian Perspective
Meenal Garg1, Shilpa D Kulkarni1, Anaita Udwadia Hegde1
1Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India.
Background:
Myasthenia Gravis is an autoimmune disorder of the neuromuscular junction which is divided into three categories in children: neonatal or transient, congenital myasthenic syndromes, and autoimmune or juvenile myasthenia gravis (JMG). JMG differs from adult MG in many aspects. Characteristics of JMG also vary between children of various ethnic groups.
Objective:
Although common, there is scant data about JMG in India. This study was performed to determine the characteristics of JMG in Indian children.
Materials And Methods:
We did a retrospective chart analysis of all patients who received a diagnosis of myasthenia at a tertiary care pediatric hospital in Mumbai, India from January 2005 to December 2015. We studied the demographic data, clinical features, investigations, history of crises, and records of hospital stay. Treatment modalities and outcomes were reviewed.
Results:
Fifteen children received a diagnosis of JMG during the study period. This was a pre-pubertal cohort with a mean age of 3.7 years. Nine patients had generalized symptoms at presentation and six had ocular presentation. The seropositivity rate was 73%. Patients were treated with a combination of pyridostigmine, steroids, and additional immunosuppression. Nearly half the patients needed second-line immunosuppression. Four children underwent thymectomy.
Conclusion:
MG in Indian children is a severe disease with high rates of seropositivity and a relatively better outcome if identified and treated appropriately. Thymectomy has an important role in management but requires more scrutiny.
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